Anomaly of Systemic Venous Drainage With Left-to-Right Intracardiac Shunts.

A 4-YEAR-OLD boy (weight: 10.3 kg [less than third centile; height: 99 cm]) was referred to the authors’ institution because of an incidental finding of a systolic murmur during an evaluation for poor weight gain. The arterial oxygen saturation was normal. Transthoracic echocardiography revealed situs solitus (uninterrupted inferior vena cava to the left with the stomach to the right), levocardia, atrioventricular and ventriculoarterial concordant connections, severe stenosis of the right upper pulmonary vein (pressure gradient peak: 17 mmHg; mean: 12 mmHg), and a dilated left atrium and left ventricle (Fig 1, A-D). There was a perimembranous ventricular septal defect (∼12 mm). The anatomy in the chest radiograph and a computed tomography image confirmed the normal situs (Fig 2, A and B). Fig 2Chest radiograph showing normal bronchial anatomy (A) and the computerized tomography image also confirming the normal bronchial anatomy and situs solitus (B). View Large Image Figure Viewer Download Hi-res image